MPN, MDS, Histiocytic

Question 1

Most common abnormality in MDS

Answer 1

Monosomy 7 (then Trisomy 8 or 21)

Question 2

RCC criteria

Answer 2

Peripheral blasts < 2% and/or BM blasts < 5%

Question 3

RAEB criteria

Answer 3

serum blasts 2-19% and/or BM blasts 5-19%

Question 4

Mutations in JMML

Answer 4

Ras pathway - NRAS, KRAS, NF1, PTPN11 (Noonan), SHP-2, CBL

Question 5

High risk areas for LCH

Answer 5

Liver, bone marrow and spleen

Question 6

CNS LCH risk sites

Answer 6

pituitary, mastoid, orbital sphenoid, and temporal bone lesions

Question 7

HLH associated genes

Answer 7

PRF1, UNC13D, STX11, STXBP2, RAB27A, AP3B1